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Case Report A case report on epithelioid inflammatory myofibroblastic sarcoma in the abdominal cavity


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Received June 18, 2019; Accepted August 27, 2019; Epub October 1, 2019; Published October 15, 2019

Abstract: Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare entity and a novel variant of the in -flammatory myofibroblastic tumor (IMT). We report the occurrence and specific characteristics of EMIS in an adult woman. Eleven months after the operation, the patient had a recurrence and multiple metastases in the abdominal cavity. Since the tumor was spreading all over the abdominal cavity and ALK staining of the tumor was positive, crizotinib was suggested as adjuvant therapy. But she failed to respond to the crizotinib treatment and died of organ failure three months later.

Keywords: Epithelioid inflammatory myofibroblastic sarcoma, ALK, crizotinib


Inflammatory myofibroblastoma (IMT) is an

interstitial neoplasm composed of myofibro

-blast spindle cells in a mucoid stroma. Its

inflammatory infiltration mainly consists of

plasma cells and lymphocytes, occasionally

mixed with eosinophils and neutrophils [1].

Epithelioid inflammatory myofibroblastic sarco

-ma (EIMS) is a rare entity and a novel variant of

the inflammatory myofibroblastic tumor (IMT),

as it has similar malignant characteristics and

mainly consists of round‑to‑epithelioid cells [2]

with a pattern of nuclear membrane or perinu

-clear immunostaining for ALK receptor tyrosine

kinase (hereafter ALK). EMIS is a rare disease.

To the best of our knowledge, only 30 cases

have been reported in the English-language

medical literature. Herein, we report the

occur-rence and specific characteristics of EMIS in an

adult woman, and the relevant literature is

reviewed. Our report provides further informa

-tion on EMIS.

Case report

The report involves a 46‑year‑old female who

was hospitalized due to left upper abdominal

pain lasting for 3 months and abdominal dis

-tention lasting for 2 months. A CT examination

showed a solid mass in the left upper intra‑

abdomen, which could not be distinguished

from the stomach. When a strengthening scan

was performed, the mass showed non‑uniform

intensification (

Figure 1A



). A few irregu

-lar nodules were seen below the mass, and

some were fused into a mass. The larger one

was about 5.6 cm×4.0 cm×5.2 cm in size. The

mass also showed non‑uniform intensification

when a strengthening scan was performed.

A laparotomy indicated that a tumor was detect

-ed in the left upper intra‑abdomen with a size of

11 cm×6.5 cm×7 cm. It was nodular,

encapsu-lated, and with moderate hardness and gray‑

white in the cut surface. Microscopically, the

tumor cells were spindle-shaped or round like,

with mitotic figures of 4/10 HPF. There was a

large number of inflammatory cells infiltrated in

the background, with more collagen bundles,

and pleomorphic hyalinizing angiectasia. A

small area of mucoid degeneration was found

(left inferior phrenic). Multiple lymph nodes

were involved by the tumor (

Figure 2

). Immu-

[image:2.612.89.527.72.221.2] [image:2.612.92.524.277.473.2]

Figure 1. Computed tomography (CT) revealing a solid mass in the left upper intra‑abdomen (A). When a strengthen -ing scan was performed, the mass showed non‑uniform intensification (B).

Figure 2. Grossly, the huge tumor located in the left upper intra‑abdomen, nodular, encapsulated, and with moder -ate hardness and gray‑white in the cut surface. Microscopically, the tumor cells are spindle‑shaped or round like.


approximately 20%. CD56, S‑100, CDla, NF,

NSE, Calponin, CD21, CD23, CD117, CD34, and

Dog‑1 were negative. This primary pathology

finding provided a convincing diagnosis of the

inflammatory myofibroblastic tumor (

Figure 3


The tumor showed a sheet and nodular growth

pattern, and the stromal mucus changed to-

gether with prominent infiltrated lymphocytes,

neutrophils and eosinophils.

The patient did not have any additional treat

-ment after the operation. Eleven months after

the operation, because of abdominal pain, she

received CT imaging, which displayed a recur

-rent intra-abdominal tumor, and multiple me-

tastases were observed in her liver, ventral

wall, abdominal cavity, pelvic cavity, and uter

-ine appendages (

Figure 4

). Since the tumor

was spreading all over the abdominal cavity

and ALK staining of the tumor was positive

(2p23 ALK gene rearrangement by immunohis

-tochemistry), crizotinib was suggested as adju

-vant therapy. Then crizotinib (200 mg) was

administered to the patient twice a day orally.

The patient’s previous clinical symptoms were

significantly relieved, and the response evalua

-tion reached a partial response (PR) (

Figure 5


However, two months later, a CT examination

revealed the progression of the disease (



). The patient failed to respond to the treat

-ment with crizotinib. Accordingly, there was lit

-tle guidance on how to treat EIMS after pro‑

gression on crizotinib. According to the results

of genetic testing, antiangiogenic therapy may

be effective. Thus, we started treatment of giv

-ing anlotinib 12 mg qd for the full two weeks,

and three weeks for a period. Unfortunately, the

antivascular therapy achieved little effect. Due

to the poor general condition of the patient, the

follow‑up treatment gave priority to symptom


-atic supportive treatment. The patient died of

organ failure three months later.

Figure 4. The tumor recurrence eleven months after the operation, and multiple metastases are observed in the liver, ventral wall, abdominal cavity, pelvic cavity, and uterine appendages.



In 2011, Marino Enriquez et al. [2] described in

detail the clinicopathological,

immunohisto-chemical and genetic characteristics of 11

additional cases and named them EIMS to

highlight both the distinct morphology and

malignant behavior of this aggressive form of

IMT. Clinically, EIMS mainly occurs among chil

-dren and adolescents, although the overall age

range varies greatly. Previous studies reported

that the patients’ ages ranged from 7 months

to 65 years, with an average age of 33.4 years

[2‑11]. As a rare malignant mesenchymal neo

-plasm, it is often found in the lung, abdominal/

pelvic and retroperitoneal cavity, regardless of

age [10].

In clinical practice, EIMS is a rapidly growing

abdominal mass or thoracic nodule, which

attracts medical attention when abdominal

pain, ascites or pleural effusion occur. Cases of

reported EIMS have ranged in size from 6 to 26

cm [2, 5, 12]. In some cases, general fatigue

and weight loss are the primary clinical mani

-festations instead of abdominal pain and a pal

-pable nodular lesion [13].

Since cases of EIMS are so rare, the diagnosis

should be only made through a strict histologi

-cal and clini-cal manifestation. Based on the

previously reported cases, the common histo


-logical features of EIMS are abundant myxoid

stroma with inflammatory infiltrating and plump

round‑to‑epithelioid cell morphology. In most of

the cases, frequent immunopositivity for ALK

[2, 5, 12] and the RANBP2‑ALK fusion gene

[13] have also been identified.

ALK is a receptor tyrosine kinase gene located

on chromosome 2p23. The rearrangement of

the gene usually results in the high activity of

the ALK protein, which is closely related to the

expression of the ALK protein through im‑

munohistochemistry [14]. In previous reports,

RANBP2, tropomyosin 3 (TPM3), tropomyosin 4

(TPM4), clathrin heavy chain (CLTC), cysteinyl‑

tRNA synthetase (CARS), 5‑aminoimidazole‑

4‑carboxamide ribonucleotide for myltransfer

-ase/IMP cyclohydrolase (ATIC), and SEC31L1

have been identified to fuse with the ALK gene,

and provide active promoters for the fusion

gene [15-17]. Several reports have suggested

that IMTs with RANBP2‑ALK fusion usually

exhibit an epithelioid/round cell morphology

and follow a more aggressive clinical course

[18, 19]. In this case, we identified a 2p23

ALK gene rearrangement by immunohisto‑


The optimal therapy for EIMS has not been well

established. Surgical resection is considered to

be the mainstay of treatment. In view of the

postoperative adjuvant therapy, the available

experience is very limited. Most of the reported

cases were treated with post-operative

chemo-therapy or radiochemo-therapy, which seemed to have

no significant effect in controlling the rapid

recurrence [2, 3, 12, 20, 21]. Recently, an ALK

inhibitor, crizotinib, has been applied in the

treatment of EIMS with a certain effectiveness

[2, 5, 12, 21, 22].

Thus, in the present case, crizotinib (200 mg)

was administered to the patient twice a day



mechanisms of resistance in ALK‑positive

patients and found that many of the resistant

samples had ALK resistant domain mutations.

Chromophilicity is described as a resistance

mechanism. Multiple closed chain rearrange

-ments lead to the loss of the tumor suppres

-sor’s gene function and the enhancement of

carcinogenic fusion function [25]. Most muta

-tions in ALK‑positive cancers involve an ALK

tyrosine kinase domain that targets drug resis

-tance patterns [27]. In fact, each ALK tyrosine

kinase inhibitor is associated with a particular

pattern of ALK resistance mutations [26, 27].

Another less common resistance is to amplify

ALK [27].

A key problem is how to prevent the develop

-ment of drug resistance to ALK inhibitors.

Further studies are needed to be conducted

among EIMS patients. Additionally, further drug

development is imperative for both patient life

expectancy and disease remission. PD‑L1

expression in tumor cells is considered to be a

predictive index of the tumor response to immu

-nomodulatory therapies targeting the PD‑1/

PD‑L1 pathway [28], as staining for the pro

-grammed death‑ligand 1 (PD‑L1) is diffusely

positive in the case of EIMS [29].

The expression of PD‑L1 in EIMS indicates an

immune check-point blockade, representing a

novel anti‑EIMS therapy.

Disclosure of conflict of interest


Address correspondence to: Zhiguang Wang, De- partment of Oncology, The First Affiliated Hospital of Guangxi University of Chinese Medicine, No 89‑9 Dongge Road, Nanning 530000, Guangxi, China. Tel: +86‑18587904369; E‑mail: a1860@126.com


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Figure 1. Computed tomography (CT) revealing a solid mass in the left upper intra‑abdomen (A)
Figure 4. The tumor recurrence eleven months after the operation, and multiple metastases are observed in the liver, ventral wall, abdominal cavity, pelvic cavity, and uterine appendages.
Figure 6. CT examination reveals the progression of the disease (PD) three months after crizotinib.


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